Typical Kawasaki disease
© Marchesi et al. 2015
Published: 30 September 2015
Kawasaki disease (KD) was first described in Japan in 1967 by Tomisaku Kawasaki . It is an acute, systemic, self-limited vasculitis, whose targets are medium diameter vessels in each organ and apparatus. It is a vasculitis of unknown aetiology, probably multifactorial, that occurs predominantly in infants and young children. The most important complications are coronary arteries aneurysms or ectasia, that develop in 15% to 25% of untreated patients, but only in 5% of patients treated with immunoglobulins within ten days following onset of fever [2, 3].
Diagnosis of KD is based on clinical criteria [3, 4]: there are neither typical clinical features nor diagnostic tests. An increasing number of children is reported in literature, having coronary artery aneurysms or ectasia, but who do not fulfill all diagnostic criteria. These cases are defined as incomplete KD and atypical KD.
fever = 5 days associated with = 4 diagnostic criteria, even before performing echocardiography
fever = 5 days associated with < 4 diagnostic criteria and possible coronary arteries lesions
fever at fourth day with = 4 diagnostic criteria and possible coronary arteries lesions.
Cardiac: coronaritis, pericarditis, myocarditis, endocarditis, mitral regurgitation, aortic and tricuspidal regurgitation (in the acute phase), aortic dilatation (in the later phase), cardiac failure, shock, arrhythmia, coronary artery involvement (in the sub acute phase)
Vascular: Raynaud phenomenon, peripheral gangrene
Joints: arthritis or arthralgia
Nervous System: irritability, aseptic meningitis, sensor-neural hearing loss, transient unilateral peripheral facial nerve palsy
Gastrointestinal: diarrhoea, vomiting, abdominal pain, acute surgical abdomen, hepatic enlargement and jaundice, acute acalculous distention of the gallbladder (hydrops)
Urinary: aseptic pyuria, urethritis, testicular swelling
Skin: erythema and induration at the site of a previous vaccination with Bacille Calmette-Guérin, Beau lines
Respiratory: cough, rhinorrea, pulmonary nodules and infiltrates.
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